Primary sclerosing cholangitis is a rare disease that affects mainly bile ducts in and out of the liver. It can also occur in the ducts of the gallbladder and pancreas.
In cases of primary sclerosing cholangitis (PSC), the bile duct walls get inflamed, resulting in scarring and hardening. Such shifts involve narrowing of the ducts, making it hard for bile to pass into them.
Bile accumulates in the liver as a result, where it kills liver cells. Bile gradually sinks into the bloodstream.
The liver develops cirrhosis after sustaining long-term damage which is a hardening, or fibrosis. It can’t work properly any more.
PSC is closely associated with chronic ulcerative colitis, Crohn’s disease and colon cancer, but it can also occur alone.
For males, who make up 70 percent of people with PSC, the disorder is more likely to develop. Symptoms appear, on average, in males at age 40 and females at age 45.
What is primary sclerosing cholangitis?
The liver produces bile, which is a greenish-brown fluid needed for digestion.
In the liver, hepatic cells excrete bile into small tubes called bile ducts. Those tubes fall together like a leaf’s veins.
These tiny tubes drain the bile into a typical duct, a larger tube that leads into the stomach. The bile supports digestion there, and gives the brown color to the stool.
In a person with PSC inflammation and scarring contribute to blockage of the bile ducts.
Bile flows into the liver as a result. It damages liver cells over time and causes fibrosis, or cirrhosis, that is liver scarring.
When cirrhosis progresses, more scar tissue builds up, and eventually the liver becomes incapable of functioning. The scar tissue may prevent the bile ducts from draining, which can cause infection in the ducts.
Symptoms
Many people with early stage PSC do not experience any symptoms. A doctor can detect the illness only during a routine examination. A blood test may point to high levels of liver enzymes, particularly alkaline phosphatase.
Symptoms occur for the following reasons:
- Bile is not draining properly through the ducts.
- The liver is damaged.
- The liver is starting to fail.
If the bile ducts become infected, it can cause chills, fever, and upper abdominal tenderness.
Itching may occur when bile seeps into the bloodstream.
As the disease progresses, a person may develop:
Jaundice causes the skin and the whites of the eyes to become yellowish. It results in accumulation of a compound called bilirubin in the body.
An individual in the advanced stages of cirrhosis in the abdomen and feet can experience severe swelling.
Liver failure may occur 10–15 years after a person is diagnosed with PSC, or it may take longer, depending on the person.
- a deficiency of the fat-soluble vitamins A, D, E, and K
- portal hypertension
- metabolic bone diseases
- cancers of the bile duct or colon
Causes
Scientists really don’t know what triggers PSC. A mixture of environmental and genetic factors potentially play a role according to Genetics Home Reference.
Several investigators have indicated that PSC could be an inflammatory disease. These disorders come about as the immune system ‘s working changes.
The immune system usually defends the body from infections caused by foreign pathogens, such as bacteria and viruses.
However, often the immune system incorrectly identifies organs or other body parts as threats, causing certain areas to be invaded and destroyed by the body.
Nevertheless, it remains unclear exactly how an autoimmune disorder could cause PSC.
However, most autoimmune disorders affect women more frequently than men but PSC is more common among men.
Scientists also report that PSC is not responding well to a treatment known as immunosuppression, unlike autoimmune conditions.
Such issues show that it behaves differently from other autoimmune disorders if PSC is an autoimmune disease.
PSC also occurs in people with some form of inflammatory bowel disease, such as Crohn’s disease or ulcerative colitis in particular. This is also normal in people suffering from sarcoidosis, chronic pancreatitis, or other autoimmune disorders.
Genetic factors may play a role too. The disease appears to grow in clusters of family members according to Genetics Home Reference.
Researchers did not connect PSC with the hepatitis-causing viruses.
Diagnosis
If an individual has a history of inflammatory bowel disease or blood tests show any odd results, a doctor may suspect PSC.
Endoscopic retrograde cholangiopancreatography (ERCP)
Doctors commonly use the ERCP technique to diagnose and treat bile duct and pancreatic problems. A mixture of upper gastrointestinal endoscopy and X-rays is involved.
The doctor inserts a lightweight , flexible endoscope through the mouth, down the esophagus, through the stomach, and into the small intestine while the individual is under anesthesia. They then insert a thin tube into the bile ducts, through the aperture. The doctor does this to administer a dye that will clearly display on an X-ray the condition of the bile ducts.
The bile ducts appear narrower than normal in a person with PSC, and they have a beaded appearance, with several narrowed and expanded segments.
Liver biopsy
When the disease progresses, the patient can need a biopsy to assess how much damage there has been.
For microscopic examination , the doctor must apply a local anesthetic and insert a slender needle through the right lower chest region to remove a small piece of the liver.
Colonoscopy
Those with PSC are particularly likely to develop ulcerative colitis and people with both PSC and ulcerative colitis are at greater risk of developing colon cancer.
It ‘s important for that reason to have colonoscopies. They can help the doctor diagnose ulcerative colitis and detect cancer or precancerous conditions early.
Treatment
Treatment includes ursodiol, or ursodeoxycholic acid, a bile acid which the liver naturally produces.
Evidence shows that in people with PSC, it can reduce the elevated liver enzyme levels. It does not, however, improve the condition of the liver or overall survival rates.
A 2011 updated research indicates that more information is needed from the medical community about the benefits and harms this procedure provides.
A doctor may also prescribe:
- medications to relieve itching, called antipruritics
- bile acid sequestrants, such as cholestyramine, to reduce cholesterol
- antibiotics to treat infections
- vitamin supplements
If an individual is also suffering from ulcerative colitis, the doctor will recommend appropriate treatment.
A low-salt diet and diuretics may help if fluid retention from cirrhosis results in swelling of the abdomen and feet.
Studies have attempted to use various immunosuppressants and antibiotics to treat PSC, but they did not impact mortality rates. Cyclosporine, tacrolimus, methotrexate, budesonide, and metronidazole are examples of such medications.
Relieving blockages
A surgeon may in some cases use an endoscopic or surgical procedure to remove major blockages in the bile ducts.
Using an endoscope, the doctor will put a tiny tube in the narrowed bile duct, with a balloon on one end. Inflating the balloon opens the duct to allow bile to flow into it once more.
Placing plastic tubing stents in the narrowed ducts will also help hold those stents intact.
Transplantation
The American Liver Foundation states that, after diagnosis, many people with PSC ultimately require liver transplants, typically around 10 years.
The success rate of transplantation has increased with improvements in surgical methods and the use of experimental medications to prevent rejection.
After a liver transplant the result for patients with PSC is outstanding. Survival levels are high at transplant centers, and after recovery, people continue to have a decent quality of life.
